Protein Name:
Methylmalonic aciduria type A homolog, mitochondrial
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UniprotKB/SwissProt ID: Q8C7H1 (Q8C7H1)
Gene Name:
Mmaa
Organism: Mus musculus (Mouse)
Function: GTPase, binds and hydrolyzes GTP (By similarity). Involved in intracellular vitamin B12 metabolism, mediates the transport of cobalamin (Cbl) into mitochondria for the final steps of adenosylcobalamin (AdoCbl) synthesis (By similarity). Functions as a G-protein chaperone that assists AdoCbl cofactor delivery from MMAB to the methylmalonyl-CoA mutase (MMUT) (By similarity). Plays a dual role as both a protectase and a reactivase for MMUT (By similarity). Protects MMUT from progressive inactivation by oxidation by decreasing the rate of the formation of the oxidized inactive cofactor hydroxocobalamin (OH2Cbl) (By similarity). Additionally acts a reactivase by promoting the replacement of OH2Cbl by the active cofactor AdoCbl, restoring the activity of MMUT in the presence and hydrolysis of GTP (By similarity)
Other Modifications: View all modification sites in dbPTM
Protein Subcellular Localization: Mitochondrion. Cytoplasm
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Graphical Visualization of S-nitrosylation Sites:
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The S-nitrosylation sites of Q8C7H1
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| No. |
Position |
S-nitrosylated Peptide |
Secondary Structure of S-nitrosylated Peptide |
Solvent Accessibility of nitrosylated Site |
PubMed ID |
| 1 |
181 |
LSVLAVDPSS C TSGGSLLGDK |
CCHHHHEECC C HHHHHHHHCC |
4.05% |
21278135
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